1/111. E.N.T. manifestations of Von Recklinghausen's disease.Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.- - - - - - - - - - ranking = 1keywords = airway (Clic here for more details about this article) |
2/111. The outcome after perinatal management of infants with potential airway obstruction.Masses in the head and neck are being detected prenatally with increasing frequency, necessitating the need for management of potential upper airway obstruction at delivery. Establishment of the airway at delivery and its maintenance thereafter are critical. This should optimally be performed with the baby still attached to the placental circulation. The importance of multidisciplinary team management, including a high risk obstetrician, neonatologist, pediatric otolaryngologist, pediatric thoracic surgeon, and an anesthetist, cannot be overemphasized. Endotracheal intubation is attempted first, if unsuccessful then is followed by insertion of a rigid bronchoscope. tracheotomy should be reserved for airway obstructions, which are not amenable to endotracheal intubation or in babies in whom exchange from a bronchoscope to endotracheal tube cannot be safely performed. The management of six infants with prenatally diagnosed potential airway obstruction is presented. morbidity and mortality still ultimately depend on the severity of the existent anomalies.- - - - - - - - - - ranking = 69.977375346781keywords = airway obstruction, airway, obstruction (Clic here for more details about this article) |
3/111. Percutaneous dilatational tracheostomy in a patient with thyroid cancer and severe airway obstruction.A patient with extensive metastatic thyroid cancer scheduled for palliative tracheostomy is presented. He had laryngeal dislocation with severe airway obstruction and few anatomical landmarks due to tumour infiltration and radiation. Successful percutaneous dilatational tracheostomy was performed under local anaesthesia.- - - - - - - - - - ranking = 49.269553819129keywords = airway obstruction, airway, obstruction (Clic here for more details about this article) |
4/111. head and neck cancers associated with Madelung's disease.BACKGROUND: Madelung's disease is a rare lipodystrophy that presents with multiple fatty masses in the neck, trunk, and upper extremities. The fatty accumulation is considered a benign disease, but compression of the aerodigestive tract may occur in long-standing disease. methods: Eight Chinese patients with Madelung's disease were reviewed. All were male, aged 48 to 67 years, with a history of disease ranging from 4 to 20 years. Two of the eight patients developed aerodigestive symptoms and were subsequently found to have head and neck cancers. These two patients are described. RESULTS: The possible mechanism that may account for an increase in malignant tumors of the airway in this group of patients is the synergistic effect of smoking and alcohol abuse as risk factors for both Madelung's disease and malignant tumors of the airway. Currently it is recommended that these patients should have their fatty lesions removed surgically. The removal of fat facilitates examination of the neck for signs of cervical lymphadenopathy in malignant disease. CONCLUSIONS: patients with Madelung's disease should be followed regularly. The development of aerodigestive symptoms should be fully investigated with endoscopy and imaging. The cause of symptoms should not be attributed to fatty compression until a carcinoma of the upper airway has been excluded.- - - - - - - - - - ranking = 3keywords = airway (Clic here for more details about this article) |
5/111. Successful management of fetal cervical teratoma using the EXIT procedure.Fetal cervical teratoma is a cause of polyhydramnios, premature labor, and newborn airway obstruction. Formation of a multispecialty team and use of the EXIT procedure is essential for survival of the neonate. Without a team, there is little hope for fetal survival; mortality will be 80-100%. early diagnosis and planning are essential. Cervical teratomas can contribute to pulmonary insufficiency and chondromalacia because of a mass effect in utero and underdevelopment of the fetal lungs.- - - - - - - - - - ranking = 9.8539107638259keywords = airway obstruction, airway, obstruction (Clic here for more details about this article) |
6/111. proteus syndrome: craniofacial and cerebral MRI.The proteus syndrome is a rare hamartoneoplastic syndrome that may affect the brain, skull, and extracranial head and neck. We present a case with severe, characteristic findings. Brain abnormalities are not common in proteus syndrome; when present, hemimegalencephaly and migrational disorders are typically seen, commonly with an associated seizure disorder. Maxillary and mandibular dysmorphism may occur, including unilateral condylar hyperplasia. Subcutaneous fatty, fibrous, lymphangiomatous masses commonly seen in this syndrome may involve the neck and face, leading to disfigurement and potential airway compromise.- - - - - - - - - - ranking = 1keywords = airway (Clic here for more details about this article) |
7/111. prenatal diagnosis of airway compromise: EXIT (ex utero intra-partum treatment) and foetal airway surgery.Four cases of potential airway obstruction diagnosed in the early antenatal period are presented. Their management is outlined, and the need for a multidisciplinary team approach to these problems is highlighted. The experience of the ex-utero intrapartum treatment (EXIT) procedure is presented, and the first attempt at intra-uterine tracheal surgery is introduced.- - - - - - - - - - ranking = 17.853910763826keywords = airway obstruction, airway, obstruction (Clic here for more details about this article) |
8/111. The ex utero intrapartum treatment procedure for a large fetal neck mass in a twin gestation.BACKGROUND: Large fetal neck masses can make it difficult or impossible to secure airways at birth, with associated risks of hypoxia, brain injury, and death. Based on a medline search from 1966 to June 1998, using the keywords EXIT procedure, placental support, twins, and neck mass, we report the first ex utero intrapartum treatment procedure performed in a twin gestation complicated by a large fetal neck mass. CASE: A giant fetal cervical mass was diagnosed in one fetus of a 20-week twin gestation by sonography and magnetic resonance imaging. At 35 weeks' gestation, the ex utero intrapartum treatment procedure was performed successfully for delivery of the normal twin, followed by intrapartum airway access of the twin with the neck mass. CONCLUSION: Even in twin gestations, the ex utero intrapartum treatment procedure is the delivery method of choice for fetuses with giant neck masses.- - - - - - - - - - ranking = 2keywords = airway (Clic here for more details about this article) |
9/111. head and neck teratomas in children.A retrospective review of seven patients with teratomas of the head and neck treated at out center over the past 5 years, which represented 2% of all teratomas (sacrococcygeal, ovarian, and retroperitoneal) seen over the past 20 years, was performed. After investigation to exclude associated anomalies, all but one of the children underwent surgery for removal of the tumour. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. The patients were followed up at regular intervals for up to 4 years. The patients' ages ranged from newborn to 2.5 years. There were three cervical, two oral, and two skull teratomas. The youngest patient had a cervical teratoma with respiratory compromise, requiring tracheal intubation. All but one patient (skull teratoma) had excision of the tumours with a satisfactory outcome. Histopathological examination of the excised tumours showed mature tissue from the three germinal layers in all specimens. Specific components included glandular epithelium, keratinising epithelium, and muscle fibres. Follow-up did not show any recurrence in the operated children. The three with cervical teratomas had normal levels of thyroid hormone postoperatively. head and neck teratomas in children are mostly benign lesions amenable to curative excision. In cervical teratomas airway management takes priority.- - - - - - - - - - ranking = 1keywords = airway (Clic here for more details about this article) |
10/111. Congenital cervical teratoma in neonates. Case report and review.Cervical teratomas are uncommon neoplasms. Although these lesions are histologically benign they are usually large and may cause airway obstruction. Cervical teratomas are usually diagnosed at birth. In-utero diagnosis is possible by prenatal ultrasound which assists in planning early airway management and surgical intervention. mortality is significant but prognosis is good with airway control and complete surgical excision. However, pressure injury of contiguous structures can limit resectability and adversely affect outcome. Malignant cervical teratoma with metastasis has been reported mostly arising in adults with poor outcome. We present nine cases of neonatal cervical teratoma identified at two institutions between 1984 and 1996. One patient died before surgical intervention. All others underwent resection. There was one intraoperative death and one postoperative death. The remaining six patients did well postoperatively with no significant sequelae with 3 to 14 years follow-up.- - - - - - - - - - ranking = 11.853910763826keywords = airway obstruction, airway, obstruction (Clic here for more details about this article) |
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